Gastrointestinal and Liver Tumors
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1 Gastrointestinal and Liver Tumors
2 Springer-Verlag Berlin Heidelberg GmbH
3 w. SCHEPPACH. R.S. BRESALIER G.N.J. TYTGAT (EDS.) Gastrointestinal and Liver Tumors Springer
4 Cataloging-in-Publication Data applied for A catalog record for this book is available from the Library of Congress. Bibliographic information published by Die Deutsche Bibliothek Die Deutsche Bibliothek lists this publication in the Deutsche Nationalbibliografie; detailed bibliographic data is available in the Internet at ISBN ISBN (ebook) DOI / This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broad-casting, reproduction on microfilm or in any other way, in storage in data banks. Duplications of this publication or parts thereof is permitted only under the provision of the German Copyright Law of September 9, 1965, in its current version, and permission for use must always be obtained from Springer-Verlag. Violations are liable for prosecution under the German Copyright Law. Springer-Verlag Berlin Heidelberg 2004 Originally published by Springer-Verlag Berlin Heidelberg New York in 2004 The use of general descriptive names, registered names, trademarks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. Product liability: The publisher cannot guarantee the accuracy of any information about dosage and application thereof contained in this book. In every individual case the user must check such information by consulting the relevant literature. Cover Design: Frido Stein en, Spanien Typesetting: Hilger VerlagsService, Heidelberg Production Editor: Frank Krabbes, Heidelberg SPIN Printed on acid-free paper
5 Foreword Cancer of the digestive system, which includes the gastrointestinal tract, liver, pancreas and gall bladder, represents a major cause of cancer morbidity and mortality worldwide. In the year 2000, these malignancies were responsible for 30% of all cancers and 36% of all cancer-associated deaths (Parkin DM, Bray Fl, Devesa SS, Cancer burden in the year The global picture. Eur J Cancer 2001; 37: S4-66). This book provides a concise, but more than adequate overview of these malignancies and their premalignant states. It gives updated information on etiology - whether genetic or environmental, pathology, clinical features and treatments. It also gives information on screening, prevention and chemoprevention, where available. Understanding these latter features are of prime importance as we clinicians are moving from treatment of endstage disease, to prevention, chemoprevention and early diagnosis in order to reduce the cancer burden, to try and delay or at least modulate its manifestations and cure it by diagnosing in the earliest and curable stage. As physicians, we are used to emphasizing diagnosis and treatment of disease. Today we already have some of the tools needed to prevent several important prevalent gastrointestinal malignancies. These include: immunization against hepatitis B, which will significantly reduce the incidence of hepatoma; improved hygiene and nutrition to decrease the prevalence and duration of exposure and severity of infection from Helicobacter pylori so as to decrease the occurrence of gastric cancer; colon cancer incidence can be reduced in the long-term by lifestyle modulation and morbidity reduced immediately by population screening, preventive polypectomy and early cancer diagnosis. The chapter authors are authorities in their fields and have produced a text for the practicing physician who needs an easy to read and complete source of information on Digestive Cancers. This book will be an excellent source of information for the treating clinician, whether gastroenterologist, surgeon or oncologist. PAUL ROZEN, MB.BS Dept. of Gastroenterology, Tel Aviv Medical Center and Professor of Medicine, Tel Aviv University
6 Preface What can be a rationale to produce a medical textbook in the era of electronic multimedia? In the view of the editors the acquisition of information and knowledge by reading a book is still more delightful for most of us than by manoevering through PC menues. However, in order to keep up with the rapid progress in gastroenterology and hepatology, a short lag time between the writing of the individual chapters and the release of the book has to be assured. The goal of this book is to present the diagnosis and treatment of gastrointestinal and liver tumors to medical doctors in hospitals or private practices, residents and libraries with interest in high-standard education in clinical oncology. On the one hand the book should provide comprehensive in-depth information on the entire clinical management of alimentary tract tumors; the text is enriched by high-quality illustrations and artwork. On the other hand the structure of chapters should allow quick reference to a topic. Therefore, text elements such as summaries, tables and treatment algorithms were introduced to make selective reading possible. The homogeneity of the individual chapters was assured by presenting all aspects in the same order (epidemiology, screening where appropriate, symptoms and clinical signs, diagnostic studies, staging and classification, treatment, prognosis, follow-up, future perspectives). The presentation of imaging techniques was integrated into those organ chapters where they are mostly applied. A paragraph on future perspectives was added to every chapter in order to point at new diagnostic and therapeutic tools that have not yet entered the clinical stage. The final chapter of the book deals with the supportive care of cancer patients. In many of them there will be a stage when tumor-targeted therapies fail to control malignant growth. In these circumstances gastroenterologists should reassure their patients that best supportive care can be highly effective in alleviating symptoms.
7 VIII Preface Authors from more than one country with a high reputation within their respective areas reflect the international standard of the content. It was also our goal to demonstrate the interdisciplinary approach to patient care and to open new ways of cooperation between the classical disciplines of clinical medicine. With contributions by many authors, redundancy of information is unavoidable. We have not eliminated background information required to comprehend a complex topic even if it may appear in another chapter. Medical textbooks are usually not read from cover to cover like a novel; therefore we hope that remaining repetitions are not disturbing the general impression. The book is dedicated to our cancer patients in the hope for further improvements in the management of their diseases. Wiirzburg, Houston, Amsterdam, January 2003 WOLFGANG SCHEPPACH, ROBERT S. BRESALIER, GUIDO N.J. TYTGAT
8 Contents Esophagus 1 Squamous Cell Carcinoma of the Esophagus 3 S.J. SPECHLER Summary... 3 Epidemiology... 3 Etiology... 3 Symptoms and Clinical Signs... 4 Diagnostics... 4 Histological Classification and Molecular Genetics... 6 Staging... 7 Treatment... 8 Prognosis and Follow-Up... lo Future Perspectives Adenocarcinoma of the Esophagus o. PECH, A. MAY, 1. GOSSNER, C. ELL Summary Epidemiology Etiology and Pathogenesis Symptoms and Clinical Signs Diagnosis Histological Classification and Molecular Genetics Staging Treatment Prognosis Future Perspectives Other Tumors ofthe Esophagus R. LAMBERT, J.Y. SCOAZEC Summary Mesenchymal Tumors of the Esophagus Endocrine Tumors of the Esophagus Lymphoma of the Esophagus Secondary Tumors of the Esophagus Melanoma of the Esophagus
9 X Table of Contents n Stomach 4 Gastric Cancer A.T.R. AXON, M.P. DIXON, H.M. SUE-LING Summary Epidemiology Etiology and Pathogenesis Symptoms and Clinical Signs Diagnostics Histological Classification and Molecular Genetics Staging Treatment Prognosis Follow-Up Future Perspectives Lymphoma of the Stomach B.A. DRAGOSICS Summary Epidemiology Etiology and Pathogenesis Symptoms and Clinical Signs Diagnostics Histological Classification and Molecular Genetics Staging Treatment Prognosis and Follow-Up Future Perspectives Gastric Tumors Other than Adenocarcinoma and Lymphoma N.C.T. VAN GRIEKEN, G.A. MEIJER, E.J. KUIPERS Summary Epithelial Tumors Mesenchymal Tumors of the Stomach Small Intestine 7 Neoplasms of the Small Intestine K. GEBOES, C. DE WOLF-PEETERS Summary Epidemiology Etiology Symptoms and Clinical Signs Diagnostics Histological Classification and Molecular Genetics
10 Table of Contents XI Staging Treatment Prognosis and Follow-Up Future Perspectives Colon and Rectum 8 Colorectal Cancer R.S. BRESALIER, S.B. Ho Summary Epidemiology Etiology and Pathogenesis Symptoms and Clinical Signs Diagnostics Histological Classification and Molecular Genetics Staging Treatment Future Perspectives Sporadic Adenomas of the Large Intestine S.B. Ho, J.H. BOND Summary Epidemiology Etiology and Pathogenesis Symptoms and Clinical Signs Diagnostics Histological Classification and Molecular Genetics Treatment Future Perspectives Hereditary Polyposis Syndromes J.M. CARETHERS Summary Adenomatous Polyposis Syndromes Hamartomatous Polyposis Syndromes Juvenile Polyposis Syndrome (IPS) Hereditary Mixed Polyposis Syndrome (HMPS)... " Hereditary Nonpolyposis Colorectal Cancer (Lynch Syndrome) RT. LYNCH, J.P. LYNCH Summary Epidemiology Etiology and Pathogenesis Symptoms and Clinical Signs Diagnostics
11 XII Table of Contents Histological Classification and Molecular Genetics 154 Staging Treatment Prognosis Follow-Up Future Perspectives Colorectal Cancer in Inflammatory Bowel Disease V. CROOG, S.H. ITZKOWITZ Summary Epidemiology Etiology and Pathogenesis Symptoms and Clinical Signs Diagnostics Histological Classification and Molecular Genetics Staging Treatment..., Prognosis and Follow-Up Future Perspectives Anal Canal 13 Anal Carcinoma 169 J.C. EGGENBERGER Summary Epidemiology Etiology and Pathogenesis Symptoms and Clinical Signs Diagnostics Histological Classification and Molecular Genetics Staging Treatment Prognosis Follow-Up Future Perspectives Pancreas 14 Neoplastic Lesions of the Pancreas 181 H.G. BEGER, M. SIECH Summary Adenocarcinoma of the Pancreas Cystic Carcinoma of the Pancreas Other Tumors
12 Table of Contents XIII Neuroendocrine Gastro-Entero-Pancreatic Tumors 15 Neuroendocrine Gastro-Entero-Pancreatic (GEP) Tumors Ro ARNOLD, Ro GOKE, Mo WIED, THo BEHR Summary Definition Classification Epidemiology Etiology General Pathophysiology Histopathology Molecular Pathogenesis Growth Characteristics and Metastatic Spread and Secondary Non-Endocrine Malignancies Staging of Endocrine GEP Tumors Clinical Entities: Symptoms and Laboratory Findings General Aspect of Management "'" Biliary System and Papilla of Vater 16 Carcinoma of the Gallbladder and Bile Ducts K. SCHOPPMEYER, K. CACA, Jo MOSSNER Summary Epidemiology Etiology Symptoms and Clinical Signs Diagnostics Histological Classification and Molecular Genetics Staging Treatment Prognosis and Follow-Up Future Perspectives Benign Tumors of the Biliary System K. SCHOPPMEYER, K. CACA, Jo MOSSNER Summary Epidemiology Etiology Symptoms and Clinical Signs Diagnostics Histological Classification and Molecular Genetics Staging Treatment Prognosis and Follow-Up Future Perspectives
13 XIV Table of Contents 18 Malignant Tumors of the Ampulla of Vater W. SCHEPPACH, H.-J. GASSEL, J. G. MUELLER Summary Epidemiology Etiology Symptoms and Clinical Signs Diagnostics Histological Classification and Molecular Genetics Staging Treatment Prognosis and Follow-Up Future Perspectives Liver 19 Hepatocellular Carcinoma M. SCHEURLEN, H.-J. BRAMBS, H.-J. GASSEL Summary Epidemiology Etiology and Pathogenesis Symptoms and Clinical Signs Diagnostics Histological Classification Staging Treatment Prognosis Follow-Up Future Perspectives Colorectal Liver Metastases H.-J. GASSEL, M. GASSER, A.M. WAAGA-GASSER, W. TIMMERMANN Summary Epidemiology Etiology and Pathogenesis Symptoms and Clinical Signs Diagnostics Histological Classification and Molecular Genetics Treatment Prognosis Follow-Up Future Perspectives
14 Table of Contents XV 21 Liver Metastases of Noncolorectal Cancer W. TIMMERMANN, R. KELLERSMANN, H.-J. GASSEL Summary Epidemiology Symptoms and Clinical Signs Diagnostics Histological Classification and Molecular Genetics Treatment Prognosis and Follow-Up Future Perspectives Appendix (w. SCHEPPACH) Benign Liver Tumors E. BlECKER, H.P. FISCHER, H. STRUNK, T. SAUERBRUCH Summary Epidemiology Etiology Symptoms and Clinical Signs Diagnostics Histological Classification and Molecular Genetics Treatment Appendix 23 Supportive Care of Patients with Gastrointestinal and Liver Tumors W. SCHEPPACH Summary Nutritional Support Analgesic Therapy Antiemetic Therapy Management of Malignancy-Related Ascites Management of Bowel Obstruction Due to Peritoneal Carcinomatosis End-of-life Care Addendum Index Calculation of Body Surface Area
15 List of Contributors ARNOLD, R., Philipps-Universitat Marburg, Klinik flir Innere Medizin, Abteilung flir Gastroenterologie und Endokrinologie, BaldingerstraBe, Marburg, Germany AXON,A.T.R., The General Infirmary, The Leeds Teaching Hospitals, Gastroenterology Unit, Great George Street, Leeds, LSI 3EX West Yorkshire, UK BEGER, H.G., Universitat Ulm, Chirurgische Universitatsklinik u. Poliklinik, SteinhoevelstraBe 9, Ulm, Germany BEHR, T., Philipps-Universitat Marburg, Klinik flir Innere Medizin, Abteilung flir Gastroenterologie und Endokrinologie, Abteilung flir Nuklearmedizin, BaldingerstraBe, Marburg, Germany BlECKER, E., Rheinische Friedrich-Wilhelms-Universitat, Zentrum flir Innere Medizin, Sigmund-Freud-StraBe 25,53127 Bonn, Germany BOND, J.H., University of Minnesota, VA Medical Center, Minneapolis, MN 55417, USA BRAMBS, H.-J., Universitat Ulm, Radiologische Universitatsklinik, Abteilung flir Diagnostische Radiologie, SteinhiivelstraBe 9, Ulm, Germany BRESALlER, R.S., M.D. Anderson Cancer Center, 1515 Holcombe Boulevard - Unit 436, Houston, TX 77030, USA CACA, K., Universitatsklinikum Leipzig, Medizinische Klinik und Poliklinik II, Philipp-Rosenthal-StraBe 27, Leipzig, Germany CARETHERS, J.M., VA San Diego Healthcare System, GI Section, MC 9-11lD, 3350 La Jolla Village Drive, San Diego, CA 92161, USA CROOG, V., Mount Sinai School of Medicine, The Dr. Henry D. Janowitz Division of Gastroenterology, One Gustave Levy Place, New York, NY 10029, USA DE WOLF-PEETERS, C., University Hospital KUL, Dpt. of Morphology and Molecular Pathology, Minderbroedersstraat 12, B-3000, Leuven, Belgium DIXON, M.F., University of Leeds, Academic Unit of Pathological Sciences, LS2 9JT Leeds, UK DRAGOSICS, B.A., Wiener Gebietskrankenkasse, Gesundheitszentrum Wien Slid, Wienerbergstrasse 13, 1100 Wien, Austria EGGENBERGER. J.C., Henry Ford Hospital, Division of Colon & Rectal Surgery, Detroit, Michigan 48202, USA ELL, c., HSK Dr.-Horst-Schmidt-Kliniken GmbH, Klinik Innere Medizin II, Ludwig Erhard-Str. 100,65199 Wiesbaden, Germany FISCHER, H.P., Rheinische Friedrich-Wilhelms-Universitat, Pathologisches Institut, Sigmund-Freud-StraBe 25, Bonn, Germany GASSEL, H.-J., Bayerische Julius-Maximilians-Universitat, Chirurgische Universitatsklinik, Josef-Schneider-StraBe 2,97080 Wlirzburg, Germany GASSER, M., Bayerische Julius-Maximilians-Universitat, Chirurgische Universitatsklinik, Josef-Schneider-StraBe 2,97080 Wlirzburg, Germany GEBOES, K., Universitaire Ziekenhuizen Leuven, Pathologische Ontleedkunde II, Minderbroedersstraat 12, 3000 Leuven, Belgium GOKE, R., Philipps-Universitat Marburg, Klinik flir Innere Medizin, Abteilung flir Gastroenterologie und Endokrinologie, BaldingerstraBe, Marburg, Germany GOSSNER, 1., HSK Dr.-Horst-Schmidt-Kliniken GmbH, Klinik Innere Medizin II, Ludwig-Erhard-Str. 100,65199 Wiesbaden, Germany
16 XVIII List of Contributors Ho, S.B., VA Medical Center, GI Section (llld), 1 Veterans Drive, Minneapolis, MN 55417, USA ITZKOWITZ, S.H., Mount Sinai School of Medicine, The Dr. Henry D. Janowitz Division of Gastroenterology, One Gustave Levy Place, New York, NY 10029, USA KELLERSMANN, R., Bayerische Julius-Maximilians-Universitat, Klinik und Poliklinik fiir Chirurgie, Josef-Schneider-StraBe 2,97080 Wiirzburg, Germany KUIPERS, E.J., Erasmus MC University Medical Center, Department of Gastroenterology and Hepatology, Burgemeester Oudlaan 50, NL-3062 PA Rotterdam, The Netherlands LAMBERT, R., International Agency for Research on Cancer, 150 Cours Albert Thomas, Lyon,69008,France LYNCH, H.T., Creighton University, Hereditary Cancer Institute, Dpt. of Preventive Medicine and Public Health, 2500 California Plaza, Omaha, Nebraska 68178, USA MAY, A., HSK Dr.-Horst-Schmidt-Kliniken GmbH, Klinik Innere Medizin II, Ludwig Erhard-StraBe 100,65199 Wiesbaden, Germany MEIJER, G.A., VU Medical Center, Dpt. of Pathology, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands MOSSNER, J., Universitatsklinikum Leipzig, Medizinische Klinik und Poliklinik II, Philipp-Rosenthal-StraBe 27, Leipzig, Germany MUELLER, J.G., Bayerische Julius-Maximilians-Universitat, Pathologisches Institut, Josef-Schneider-StraBe 2, Wiirzburg, Germany PECH, 0., HSK Dr.-Horst-Schmidt-Kliniken GmbH, Klinik Innere Medizin II, Ludwig-Erhard-StraBe 100,65199 Wiesbaden, Germany SAUERBRUCH, T., Rheinische Friedrich-Wilhelms-Universitat, Zentrum fur Innere Medizin, Abt. fur Innere Medizin I, Sigmund-Freud-StraBe 25, Bonn, Germany SCHEPPACH, W., Bayerische Julius-Maximilians-Universitat, Medizinische Universitatsklinik, Schwerpunkt Gastroenterologie, Josef-Schneider StraBe 2, Wiirzburg, Germany SCHEURLEN, M., Bayerische Julius-Maximilians-UniversiUit, Medizinische Poliklinik, KlinikstraBe 8, Wiirzburg, Germany SCHOPPMEYER, K., Universitatsklinikum Leipzig, Medizinische Klinik und Poliklinik II, Philipp-Rosenthal-StraBe 27,04103 Leipzig, Germany SCOAZEC, J.Y., Hospital E. Herriot, Dpt. of Pathology, Place d' Arsonval, F-Lyon 69003, France SIECH, M., Universitat Ulm, Chirurgische Universitatsklinik und Poliklinik, SteinhoevelstraBe 9,89075 Ulm, Germany SPECHLER, S.J., The University of Texas, Dallas Veterans Affairs Medical Center, Dpt. of Internal Medicine, Gastroenterology Section (111.B 1), Division of Digestive and Liver Diseases, 4500 S. Lancaster Road, Texas 75216, USA STRUNK, H., Rheinische Friedrich-Wilhelms-Universitat, Radiologische Klinik, Sigmund-Freud-Str. 25, Bonn, Germany SUE-LING, H.M., The General Infirmary, Division of Surgery and Centre for Digestive Diseases, Leeds LSI 3EX, UK TIMMERMANN, w., Bayerische Julius-Maximilians-Universitat, Chirurgische Universitatsklinik, Josef-Schneider-StraBe 2, Wiirzburg, Germany VAN GRIEKEN, N.C. T., VU Medical Center, Dpt. of Gastroenterology, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands WAAGA-GAssER,A.M., Bayerische Julius-Maximilians-Universitat, Chirurgische Universitatsklinik, Abt. fiir Molekulare Onkologie and Immunologie, Josef-Schneider StraBe 2, Wiirzburg, Germany WIED, M., Philipps-Universitat Marburg, Klinik fiir Innere Medizin, Abteilung fiir Gastroenterologie und Endokrinologie, BaldingerstraBe, Marburg, Germany
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